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      Interstitial lung disease

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          Abstract

          Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. Knowledge achieved in recent years has resulted in the publication of the new classification of idiopathic interstitial pneumonias, according to which there are three groups: major, rare and unclassified. The novelty of the new classification comes from the fact that difficult to classify entities can be treated according to the disease behaviour classification. Idiopathic pulmonary fibrosis is the most lethal amongst the interstitial lung diseases and presents high heterogeneity in clinical behaviour. A number of biomarkers have been proposed in order to predict the course of the disease and group patients with the same characteristics in clinical trials. Early diagnosis and disease stratification is also important in the field of other interstitial lung diseases.

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          An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

          Ganesh Raghu, Harold Collard, Jim J. J. Egan … (2011)
          American Journal of Respiratory and Critical Care Medicine, 183(6), 788-824
          Article 0 comments Cited 1185 times – based on 0 reviews     Review now
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            An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias.

            William Travis, Ulrich Costabel, David Hansell … (2013)
            In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical "gold standard" of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs. The objective of this statement is to update the 2002 ATS/ERS classification of IIPs. An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011. Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment. This update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.
            Article 0 comments Cited 823 times – based on 0 reviews     Review now
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              A common MUC5B promoter polymorphism and pulmonary fibrosis.

              Max A Seibold, Anastasia Wise, Marcy Speer … (2011)
              The mutations that have been implicated in pulmonary fibrosis account for only a small proportion of the population risk. Using a genomewide linkage scan, we detected linkage between idiopathic interstitial pneumonia and a 3.4-Mb region of chromosome 11p15 in 82 families. We then evaluated genetic variation in this region in gel-forming mucin genes expressed in the lung among 83 subjects with familial interstitial pneumonia, 492 subjects with idiopathic pulmonary fibrosis, and 322 controls. MUC5B expression was assessed in lung tissue. Linkage and fine mapping were used to identify a region of interest on the p-terminus of chromosome 11 that included gel-forming mucin genes. The minor-allele of the single-nucleotide polymorphism (SNP) rs35705950, located 3 kb upstream of the MUC5B transcription start site, was present at a frequency of 34% among subjects with familial interstitial pneumonia, 38% among subjects with idiopathic pulmonary fibrosis, and 9% among controls (allelic association with familial interstitial pneumonia, P=1.2×10(-15); allelic association with idiopathic pulmonary fibrosis, P=2.5×10(-37)). The odds ratios for disease among subjects who were heterozygous and those who were homozygous for the minor allele of this SNP were 6.8 (95% confidence interval [CI], 3.9 to 12.0) and 20.8 (95% CI, 3.8 to 113.7), respectively, for familial interstitial pneumonia and 9.0 (95% CI, 6.2 to 13.1) and 21.8 (95% CI, 5.1 to 93.5), respectively, for idiopathic pulmonary fibrosis. MUC5B expression in the lung was 14.1 times as high in subjects who had idiopathic pulmonary fibrosis as in those who did not (P<0.001). The variant allele of rs35705950 was associated with up-regulation in MUC5B expression in the lung in unaffected subjects (expression was 37.4 times as high as in unaffected subjects homozygous for the wild-type allele, P<0.001). MUC5B protein was expressed in lesions of idiopathic pulmonary fibrosis. A common polymorphism in the promoter of MUC5B is associated with familial interstitial pneumonia and idiopathic pulmonary fibrosis. Our findings suggest that dysregulated MUC5B expression in the lung may be involved in the pathogenesis of pulmonary fibrosis. (Funded by the National Heart, Lung, and Blood Institute and others.).
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                Author and article information

                Journal
                Journal ID (nlm-ta): Eur Respir Rev
                Journal ID (iso-abbrev): Eur Respir Rev
                Journal ID (publisher-id): err
                Title: European Respiratory Review
                Publisher: European Respiratory Society (442 Glossop Road, Sheffield, S10 2PX, UK )
                ISSN (Print): 0905-9180
                ISSN (Electronic): 1600-0617
                Publication date (Print): March 2014
                Volume: 23
                Issue: 131
                Pages: 40-54
                Affiliations
                [1 ]Dept of Thoracic Medicine and Laboratory of Cellular and Molecular Pneumonology, Medical School, University of Crete , Crete, Greece. [2 ]Pulmonary Unit, GB Morgagni Hospital , Forlì, Italy. [3 ]Dept of Pneumology and Allergy, Ruhrlandklinik, University Hospital, University of Duisburg Essen , Essen, Germany.
                Author notes
                K.M. Antoniou, Dept of Thoracic Medicine and Laboratory of Cellular and Molecular Pneumonology, Medical School, University of Crete, Heraklion 71110 Crete, Greece. E-mail: kantoniou@ 123456med.uoc.gr
                Article
                Publisher ID: err0091-2013
                DOI: 10.1183/09059180.00009113
                PMC ID: 9487254
                PubMed ID: 24591661
                SO-VID: d4ead3e8-ae6c-42e5-9a96-689b58366844
                Copyright © ©ERS 2014
                License:

                ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 3.0.

                History
                Date received : 13 December 2013
                Date accepted : 14 January 2014
                Categories
                Subject: Clinical Year in Review

                Data availability:

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